PKD1 Antibody (YA7594)

PKD1 encodes polycystin-1 (PC1), a large transmembrane protein that functions as a receptor-like signaling molecule in renal epithelial cells and other tissues, where it integrates extracellular cues with intracellular responses involved in tissue homeostasis and morphogenesis^[1][2]. Mechanistically, PC1 forms a functional complex with polycystin-2 (PC2), and this receptor-channel assembly localizes to the plasma membrane, endoplasmic reticulum, and primary cilium, where it participates in mechanosensory and calcium-dependent signaling pathways that regulate epithelial function^[2][3]. Through its extensive extracellular domains and intracellular signaling capacity, PC1 contributes to cell-cell adhesion, cell-matrix interactions, and signal transduction processes that maintain normal tubular architecture^[4][5]. Disease relevance is highlighted by the fact that PKD1 mutations account for the majority of autosomal dominant polycystic kidney disease (ADPKD) cases, in which disruption of polycystin signaling promotes progressive renal cyst formation and loss of kidney function^[2][6]. In experimental models, loss of PKD1 alters ciliary signaling and cellular responses linked to cystogenesis, supporting a central role for PC1 in maintaining renal epithelial integrity^[3][6]. Compared with the related isoform PC2 encoded by PKD2, which functions primarily as a TRPP cation channel, PC1 acts predominantly as the receptor-like component of the polycystin complex and provides distinct extracellular sensing and signaling functions^[2][6]. For experimental applications, studies have identified GPCR-like properties of PC1, including regulation through its GPCR proteolytic site (GPS) and stalk-dependent signaling mechanisms, providing useful frameworks for mechanistic investigations of polycystin-mediated signaling pathways^[2][7].